PATHOLOGY OF VASCULAR SKIN LESIONS - PART 5 pptx

Intravascular papillary endothelial hyperplasia of the oral soft tissues: report of 18 cases and review of the literature.. Intravascular papillary endothelial hyperplasia of the mouth:

21 Heyden G, Dahl I, Angervall L Intravascular papillary endothelial hyperplasia in the oral mucosa Oral Surg Oral Med Oral Pathol 1978;45:83–7.

22 Buchner A, Merrell PW, Carpenter WM, Leider AS Oral intravascular papillary endothelial sia J Oral Pathol Med 1990;19:419–22.

hyperpla-23 Stern Y, Braslavsky D, Shpitzer T, Feinmesser R Papillary endothelial hyperplasia in the tongue: a benign lesion that may be mistaken for angiosarcoma J Otolaryngol 1994;23:81–3.

24 Tosios K, Koutlas IG, Papanicolaou SI Intravascular papillary endothelial hyperplasia of the oral soft tissues: report of 18 cases and review of the literature J Oral Maxillofac Surg 1994;52:1263–8.

25 De Courten A, Fuffer R, Samson J, Lombardi T Intravascular papillary endothelial hyperplasia of the mouth: report of six cases and literature review Oral Dis 1999;5:175–8.

26 Wehbe MA, Otto NR Intravascular papillary endothelial hyperplasia in the hand J Hand Surg [Am] 1986;11:275–9.

27 Schwartz IS, Parris A Cutaneous intravascular papillary endothelial hyperplasia: a benign lesion that may simulate angiosarcoma Cutis 1982;29:66–9.

28 Dekio S, Tsujino Y, Jidoi J Intravascular papillary endothelial hyperplasia on the penis: report of a case.

35 Katzman B, Caliguri DA, Klein DM, Nicastri AD, Chen P Recurrent intravascular papillary endothelial hyperplasia J Hand Surg [Br] 1997;22:113–5.

36 Yamamoto T, Marui T, Mizuno K Recurrent intravascular papillary endothelial hyperplasia of the toes Dermatology 2000;200:72–4.

Chapter 7 / Cutaneous Vascular Hyperplasias 123

at the site of cutaneous shunts for hemodyalisis (3–5) (Figs 14 and 15) in paralyzed extremities (6,7) and in patients with Klippel-Trenaunay syndrome (8,9) Acroangio-

dermatitis of Mali is simply exaggerated stasis dermatitis The lesions are usually eral and develop in elderly patients with chronic venous insufficiency (Fig 16) Theyhave a predilection for the dorsal aspect of the feet and ankles The lesions begin asviolaceous macules and patches that develop slowly into soft, nontender, red to purplepapules and nodules Patients also present with scaly and indurated purple plaques, and

bilat-Fig 13 Stewart-Bluefarb syndrome in the lower extremity of young male patient An underlying

arteriovenous shunt was present.

changes of stasis dermatitis are evident on the adjacent skin Lesions identical to those

of acroangiodermatitis of Mali may be seen in the distal part of an amputation stump

(10,11) (Fig 17) induced by a suction-socket prosthesis (12).

H ISTOPATHOLOGIC F EATURES

Both types of pseudo-Kaposi’s sarcoma resemble Kaposi’s sarcoma clinically, buthistopathologically they are completely different In the Mali’s variant, the histopatho-logic findings are those of stasis dermatitis, namely, there is an increased number of thick-walled vessels lined by plump endothelial cells, extravasation of erythrocytes, anddeposits of hemosiderin (Fig 18) These changes are confined to the upper half of thedermis In Stewart-Bluefarb syndrome, the entire dermis may be affected and, in largespecimens, an arteriovenous shunt may be identified Histopathologically, the differen-tial diagnosis with early stages of Kaposi’s sarcoma is usually straightforward, keeping

in mind that the patch and plaque stages of Kaposi’s sarcoma are characterized by aproliferation of irregular jagged blood vessels, which are present around preexistingvenules and adnexa and are lined by thin endothelial cells As a rule, the papillary dermis

is spared in the early stages of Kaposi’s sarcoma Recently, the expression of CD34antigen has been proposed as a feature to histopathologically distinguish lesions ofpseudo-Kaposi’s sarcoma from authentic Kaposi’s sarcoma CD34 positivity is detected

in both endothelial cells and perivascular spindle cells of Kaposi’s sarcoma, whereas no

such expression is seen in pseudo-Kaposi’s sarcoma (13) Furthermore; HHV-8 is not demonstrated in lesions of pseudo-Kaposi’s sarcoma (14).

Fig 15 Acroangiodermatitis involving the inner aspect of the forearm distally to the site of a

cutaneous arteriovenous shunt for hemodialysis The lesion showed the appearance of a ric plaque.

purpu-Chapter 7 / Cutaneous Vascular Hyperplasias 125

Fig 16 Acroangiodermatitis of Mali involving the inner aspect of the ankle of an elderly male.

Fig 17 Acroangiodermatitis of Mali involving the distal part of an amputation stump.

T REATMENT

Treatment of acroangiodermatitis of Mali is unsatisfactory and often unnecessary If

it is required, treatment of venous insufficiency of the lower extremities may be followed

by slow improvement of the cutaneous lesions Patients with Stewart-Bluefarb syndromeshould consult with a vascular surgeon in order to embolize or excise the arteriovenous

shunt under angiographic control (15).

References

1 Mali JWH, Kuiper JT, Hamers AA Acro-angiodermatitis of the foot Arch Dermatol 1965;92:515–8.

2 Bluefarb SM, Adams LA Arteriovenous malformation with angiodermatitis Stasis dermatitis ing Kaposi’s disease Arch Dermatol 1967;96:176–81.

simulat-3 Goldblum OM, Kraus E, Bronner AK Pseudo-Kaposi’s sarcoma of the hand associated with an acquired, iatrogenic arteriovenous fistula Arch Dermatol 1985;121:1038–40.

4 Landthaler M, Stolz W, Eckert F, Schmoeckel C, Braun-Falco O Pseudo-Kaposi’s sarcoma occurring after placement of arteriovenous shunt A case report with DNA content analysis J Am Acad Dermatol 1989;21:499–505.

Fig 18 Histopathologic features of acroangiodermatitis (A) Scanning magnification show

lobu-lar proliferations of capillobu-laries at the superficial dermis (B) Higher magnification demonstrates

that the lobules are composed of plump endothelial cells with extravasation of erythrocytes and deposits of hemosiderin.

Chapter 7 / Cutaneous Vascular Hyperplasias 127

5 Kim TH, Kim KH, Kang JS, Kim JH, Hwang IY Pseudo-Kaposi’s sarcoma associated with acquired arteriovenous fistula J Dermatol 1997;24:28–33.

6 Meynadier J, Malbos S, Guilhon JJ, et al Pseudo-angiosarcomatose de Kaposi sur membre paralytique Dermatologica 1980;16:190–7.

7 Landthaler M, Langehenke H, Holzmann H, Braun Falco O Akroangiodermatitis Mali Kaposi”) and gelähmten Beinen Hautarzt 1988;39:304–7.

(“Pseudo-8 Lund Kofoed M, Klemp P, Thestrup-Pedersen K The Klippel-Trenaunay syndrome with angiodermatitis (pseudo-Kaposi’s sarcoma) Acta Derm Venereol 1985;65:75–7.

acro-9 Lyle WG, Given KS Acroangiodermatitis (pseudo-Kaposi’s sarcoma) associated with Trenaunay syndrome Ann Plast Surg 1996;37:654–6.

Klippel-10 Kolde G, Wörheide J, Baumgartner R, Bröcker EB Kaposi-like acroangiodermatitis in an above-knee amputation stump Br J Dermatol 1989;120:575–80.

11 Gucluer H, Gurbuz O, Kotiloglu E Kaposi-like acroangiodermatitis in an amputee Br J Dermatol 1999;141:380–1.

12 Badell A, Marcoval J, Graells J, Moreno A, Peyri J Kaposi-like acroangiodermatitis induced by a suction-socket prosthesis Br J Dermatol 1994;131:915–7.

13 Kanitakis J, Narvaez D, Claudy A Expression of the CD34 antigen distinguishes Kaposi’s sarcoma from pseudo-Kaposi’s sarcoma (acroangiodermatitis) Br J Dermatol 1996;134:44–6.

14 Krengel S, Goerdt S, Kruger K, Schnitzler P, Geiss M, Tebbe B, Blume-Peytavi U, Orfanos CE Kaposiforme, HHV-8-negative Akroangiodermatitis bei chronisch-venoser insuffizienz Hautarzt 1999;50:208–13.

15 Utermann S, Kahle B, Petzoldt D Erfolgreiche Langzeittherapie bei Stewart-Bluefarb-Syndrom Hautarzt 2000;51:336–9.

culosis, cryoproteinemia, chronic lymphatic leukemia, hepatopathy and hypertensiveportal gastropathy, antiphospholipid syndrome, rheumatoid arthritis, dermal amyloidangiopathy, and severe peripheral vascular atherosclerotic disease, but also in patients

with no underlying disease (2–16).

Clinically, the lesions appear as red-brown or violaceous nodules or plaques over the

face (Fig 19), arms, and legs (2) In addition, petecchiae, ecchymoses, and small areas

Fig 19 Reactive angioendotheliomatosis in a patient with cryoglobulinemia Purpuric plaques on

the cheeks of an elderly woman.

Chapter 7 / Cutaneous Vascular Hyperplasias 129

of necrosis are frequently observed (3,5) The pathogenesis remains unclear, but a lating angiogenic factor has been proposed by some investigators (4,6) Wick and Rocamora (1) suggested that reactive angioendotheliomatosis is an unusual residual of

circu-leukocytoclastic vasculitis In cases associated with cryoglobulinemia or cold nins, the luminal deposits of cryoproteins may be the stimulus to induce the proliferation

aggluti-of endothelial cells (7,17) A similar pathogenesis has been proposed for glomeruloid hemangioma in POEMS syndrome (18).

Reactive intravascular angiomatosis of the skin with local deposits of intravascularimmunoglobulin resulting in a vascular proliferation with a glomeruloid pattern has alsobeen described in patients with monoclonal gammopathy and chronic lymphocytic

B-leukemia (19) In the cases of peripheral atherosclerotic disease, vascular insufficiency

from the occluded arteries appears to be the inciting factor for the endothelial proliferation,

because when the blood flow is restored by a graft bypass, the lesions resolve (8,20) We

have seen examples of both intravascular and diffuse dermal reactive theliomatosis that appeared in acral areas of the forearm and hand secondary to iatrogenicarteriovenous fistulas for hemodialysis that resolved when the arteriovenous fistula wasremoved We postulated that a local increase of vascular endothelial growth factor, as is the

angioendo-case in hypoxia, was the cause of the endothelial proliferation (21) Kunstfeld et al (22)

have recently described an example of diffuse dermal reactive angioendotheliomatosis withlesions involving the trunk in a patient undergoing chronic hemodialysis

H ISTOPATHOLOGIC F EATURES

Histopathologically, the intravascular form of reactive angioendotheliomatosis its dilated blood vessels that contain a proliferation of endothelial cells often occludingthe lumina of the vessels; occasionally there are associated fibrin thrombi (Fig 20).Focally, recanalized “glomeruloid” blood vessels are seen, especially in the cases asso-

exhib-ciated with cryoglobulinemia (7,17) Endothelial cells do not show atypia, and mitotic

figures are not identified Involved vessels are surrounded by a scanty inflammatoryinfiltrate of lymphocytes, neutrophils, and extravasated erythrocytes In the cases ofreactive angioendotheliomatosis associated with severe peripheral vascular atheroscle-rotic disease, the histopathologic picture is different In these cases the proliferation is notlocalized to preexisting vessels, or if it is, proliferation is minimal; what is more promi-nent is the presence of diffuse, interstitial proliferations of endothelial cells that percolate

between the collagen bundles of the reticular dermis (8,20–23).

Immunohistochemical studies have demonstrated that the proliferating cells in reactiveangioendotheliomatosis are endothelial cells, because they expressed factor VIII-related

antigen, Ulex europaeus I lectin, CD34, CD31, and vimentin, but they failed to express

leukocyte antigens such as leukocyte common antigen, LN2, MT1, UCHL1, and L26, as

well as epithelial membrane antigen and cytokeratins (1,7,8,18,22) In some cases,

pro-liferation of pericytic myoepithelial cells, identified by their staining with antibodies to

muscle-associated proteins, are present within and around affected blood vessels (1,9,22).

In rare instances of intravascular reactive angioendotheliomatosis, the proliferatingintravascular cells did not mark with endothelial cell markers but with markers of histio-cytic differentiation; for this type of lesion the term of intravascular histiocytosis has been

proposed (24,25) PCRs carried out in paraffin-embedded sections of reactive theliomatosis for HHV-8 DNA have been negative (22).

angioendo-T REATMENT

Cutaneous lesions of reactive angioendotheliomatosis require no treatment; most ofthem regress spontaneously when the cause is eliminated In two recently described cases

of diffuse dermal reactive angioendotheliomatosis, the lesions responded respectively to

treatment with oral methylprednisolone (22) and isotretinoin (23).

Fig 20 Histopathologic features of reactive angioendotheliomatosis (A) Low power shows

numerous vascular structures scattered at different levels of the dermis (B) Higher magnification demonstrates plump endothelial cells and fibrin thrombi occluding the lumina of the vessels (C)

Immunohistochemical studies reveal that most of the endothelial cells express immunoreactivity for CD31.

Chapter 7 / Cutaneous Vascular Hyperplasias 131

associ-A report of unusual case with immunohistochemical and immunofluorescence correlation and review

of the literature J Cutan Pathol 1999;26:206–12.

20 Kimyai-Asadi A, Nousari HC, Ketabchi N, Henneberry JM, Costarangos C Diffuse dermal tosis: a variant of reactive angioendotheliomatosis associated with atherosclerosis J Am Acad Dermatol 1999;40:257–9.

angioma-21 Requena L, Fariña MC, Renedo G, Alvarez A, Sanchez Yus E, Sangueza OP Intravascular and diffuse dermal reactive angioendotheliomatosis secondary to iatrogenic arteriovenous fistulas J Cutan Pathol 1999;26:159–64.

22 Kunstfeld R, Petzelbauer P A unique case of benign disseminated angioproliferation combining tures of Kaposi’s sarcoma and diffuse dermal angioendotheliomatosis J Am Acad Dermatol 2001;45:601–5.

fea-23 McLaughlin ER, Morris R, Weiss SW, Arbiser JL Diffuse dermal angiomatosis of the breast: response

to isotretinoin J Am Acad Dermatol 2001;45:462–5.

24 O’Grady JT, Shahidullah H, Doherty VR, Al-Nafussi A Intravascular histiocytosis Histopathology 1994;24:265-8.

25 Rieger E, Soyer HP, LeBoit PE, Metze D, Slovak R, Kerl H Reactive angioendotheliomatosis or intravascular histiocytosis? An immunohistochemical and ultrastructural study in two cases of intravas- cular histiocytic cell proliferation Br J Dermatol 1999;140:497–504.

Chapter 8 / Benign Neoplasms 133

GIANT CELL ANGIOBLASTOMA

SPINDLE CELL HEMANGIOMA (FORMERLY SPINDLE CELL

Hutchinson (1) first described angioma serpiginosum in 1889, under the term “a

peculiar form of a serpiginous and infective nevoid disease.” He used the term “infective”

to describe the pattern of progression of the disease, rather than to suggest an infectiousetiology Angioma serpiginosum is a neoplasm characterized by a proliferation of endot-helial cells and formation of new capillaries and not simply a dilation of preexisting

capillaries, as in telangiectases (2) Therefore, this lesion is included among the benign

vascular neoplasms

C LINICAL F EATURES

Clinically, the lesions of angioma serpiginosum are characterized by multiple, minute,red to purple grouped macules that extend over a period of months to years in a serpigi-

nous and gyrate patterns (3).There is no evidence of inflammation, hemorrhage, or

pig-mentation, although the purple points do not blanch completely after the application of

pressure, which could cause the misinterpretation of the lesions as purpura (4) In

doubt-ful cases, epiluminescence microscopy has been proposed as a helpdoubt-ful technique in

distinguishing angioma serpiginosum from purpuric dermatoses (5) Frequently, there is

a background of diffuse erythema The condition is asymptomatic and occurs nantly in young females, starting in childhood Most cases are sporadic, but two familiesaffected by angioma serpiginosum with an autosomal dominant inheritance have been

predomi-reported (6) The lesion has a predilection for the extremities, most frequently the lower limbs (7,8) (Fig 1), although cases involving extensive areas of the trunk and extremities have been also described (9) It is usually unilateral, at least initially, but when bilateral

involvement is present, it shows an asymmetric distribution In rare cases, the lesions may

follow the Blaschko lines (10) Occasionally, angioma serpiginosum may involve the ocular and nervous system (11) After an initial period of growth, the lesions usually remain stable in adult life, and sometimes there is partial or complete regression (12) A

group of lesions that has been described as atypical angioma serpiginosum are better

interpreted as superficial hyperkeratotic vascular malformations (13).

an inner layer consisting of a delicate fibrillary material and the outer layer composed of

collagen bundles (19,20) The presence of numerous concentrically arranged pericytes has also been described (20).

1 Hutchinson J A peculiar form of serpiginosum and infective naevoid disease Arch Surg 1889;1:275.

2 Neumann E Some new observations on the genesis of angioma serpiginosum Acta Derm Venereol 1971;51:194–8.

Fig 1 Clinical appearance of angioma serpiginosum Multiple minute red-purple grouped macules.

Chapter 8 / Benign Neoplasms 135

Fig 2 Histopathologic features of angioma serpiginosum (A) Low power shows capillary blood

vessels involving the dermal papillae (B) Higher magnification shows that these grouped

capil-lary blood vessels have thick walls.

3 Stevenson MJ, Lincoln CS Angioma serpiginosum Arch Dermatol 1967;95:16–22.

4 Cox NH, Paterson WD Angioma serpiginosum: a simulator of purpura Postgrad Med J 1991;67: 1065–6.

5 Ohnishi T, Nagayama T, Morita T, et al Angioma serpiginosum: a report of 2 cases identified using epiluminescence microscopy Arch Dermatol 1999;135:1366–8.

6 Marriott PJ, Munro DD, Ryan T Angioma serpiginosum—familial incidence Br J Dermatol 1975; 93:701–6.

7 Yaffee HS Angioma serpiginosum Arch Dermatol 1967;95:667.

8 Thiers H, Moulin G Angiome serpigineux de Hutchinson Bull Soc Fr Dermatol Syphiligr 1969;76:138.

9 Katta R, Wagner A Angioma serpiginosum with extensive cutaneous involvement J Am Acad Dermatol 2000;42:384–5.

10 Gerbig AW, Zala L, Hunziker T Angioma serpiginosum, eine Hautveranderung entlang den Linien? Hautarzt 1995;46:847–9.

Blaschko-11 Gautier-Smith PC, Sanders MD, Sanderson KV Ocular and nervous system involvement in angioma serpiginosum Br J Ophthalmol 1971;55:433–43.

12 Litoux P Angiome serpigineux (deux observations) Bull Soc Fr Dermatol Syphiligr 1969;76:54.

13 Michalowski R, Urban J Atypical angioma serpiginosum: a case report Dermatologica 1982; 164:331–7.

14 Baker LP, Sachs PM Angioma serpiginosum Arch Dermatol 1965;92:613–20.

15 Burda A, Piechocki M Uber das sogenannte Angioma serpiginosum Hautarzt 1968;19:499–504.

16 Barabasch R, Baur M Angioma serpiginosum Ein Name für verschiedene dermatologische Krankheitsbilder Hautarzt 1971;22:436–42.

17 Laugier P L’angiome serpigineux de Hutchinson Dermatologica 1967;135:369–74.

18 Reymond JL, Stoebner P, Amblard P Telangiectasies naevoides acquises Dermatologica 1979;159:489–94.

19 Kumakiri M, Katoh N, Miura Y Angioma serpiginosum J Cutan Pathol 1980;7:410–21.

20 Chavaz P, Laugier P Angiome serpigineux de Hutchinson: etude ultrastructurale Ann Dermatol Venereol 1981;108:429–36.

21 Polla LL, Tan OT, Garden JM, Parrish JA Tunable pulsed dye laser for the treatment of benign neous vascular ectasia Dermatologica 1987;174:11–7.

cuta-22 Long CC, Lanigan SW Treatment of angioma serpiginosum using a pulsed tunable dye laser Br J Dermatol 1997;136:631–2.

“mixed capillary and cavernous” components Mulliken and Glowacki (1) affirmed that

all hemangiomas, at a given point in time, show a remarkably consistent architecturalpattern throughout the entire depth of the lesion Thus the terms “capillary” and “cavern-ous” are inappropriate both clinically and histopathologically It is more appropriate todesignate bright red hemangiomas as superficial and those with normal overlying skin asdeep Hemangiomas with both superficial and deep components appear bright red in theirexophytic portions which overlie the subcutaneous nodule

malformation (2) As distinctive features, hemangiomas are rarely visible at birth, but

they appear 2–3 wk thereafter and they grow rapidly during the first weeks of life.Contrastingly, vascular malformations are usually evident at birth and enlarge commen-surate with the child’s growth As exceptions to this rule: (1) the noninvoluting congenital

hemangioma (3) is present at birth, grows proportionately with the child, and does not regress; and (2) the congenital nonprogressive hemangioma (4) is present at birth, does

not show the typical postnatal proliferative phase, and remains stable

Coloration is helpful in distinguishing a hemangioma from a vascular malformation.The bright red color of a superficial hemangioma deepens during the first year of life,whereas the hue of a vascular malformation persists unaltered Palpation is also helpful.Hemangiomas have a firm or rubbery consistency, whereas vascular malformations aresoft, easily compressible, masses Unequivocal distinction is not always possible Reex-amination after a few weeks usually resolves the problem, since rapid growth during theearly weeks of life favors the diagnosis of hemangioma As a practical matter, there israrely need for an immediate diagnosis and therapy Imaging studies are also useful.Noninvasive techniques, such as ultrasonography with Doppler studies, may disclose thehigh-flow pattern of a hemangioma which is distinct from a solid tumor or vascular

malformation (5) With computed tomography, a proliferative hemangioma appears as

a well-circumscribed homogeneous lesion, whereas a vascular malformation shows

heterogeneous densities, sometimes with calcifications and multilocular cysts (6)

Mag-Chapter 8 / Benign Neoplasms 137

netic resonance imaging (MRI) demonstrates well-circumscribed, densely lobulatedmasses with an intermediate signal intensity on T1-weighted images and a moderatelyhyperintense signal on T2-weighted images (7).

Infantile hemangiomas have a high prevalence They affect 1–3% of all neonates (8,9) and approx 10% of infants by the end of the first year of life (10,11) Evidence clearly

defines a higher incidence in premature infants, inversely related to the gestational age

at birth (12,13) The incidence of hemangioma is 10 times greater in children born of

women who had chorionic-villus sampling compared with children of women without

this maternal history (14) The incidence among twins denies hereditary factors (15) In

rare instances, however, hemangiomas are familial, and several kindreds reflect an

autosomal dominant pattern of inheritance (16).

At its inception, the lesion appears as a pink macule that enlarges to become a shaped, red to purple plaque with a smooth or lobulated surface Although they may occuranywhere on the skin, the head (Fig 3) and neck (Fig 4) are favorite locations, with thetrunk and limbs constituting sites of next frequency Not uncommonly, hemangiomasinvolve mucous membranes of the oral and genital regions A solitary lesion is the rule;however, 15–20% of involved infants manifest multiplicity There may be visceral lesions

dome-too (see below).

Infantile hemangiomas characteristically proceed through different stages: a growthphase during the early years of life, a stable period, and then characteristically, sponta-neous regression Virtually, 100% of infantile hemangiomas undergo some degree of

Fig 3 Infantile hemangioma involving the tip of the nose.

Fig 4 Ulcerated infantile hemangioma on the dorsum of the neck.

spontaneous regression (Fig 5) It has been estimated that approx 30% of infantilehemangiomas will have resolved by the third birthday, about 50% by the fifth, and about

70% by the seventh (17,18) If the hemangioma does not show signs of regression by the

time a child is 5 or 6 years of age, complete regression is unlikely Lesions that exhibit

early changes of regression typically do so more rapidly with better cosmetic results (18).

The likelihood of spontaneous regression in infantile hemangiomas is not related to theirsize, conventional anatomic location, number of aggregated lesions, or age of firstappearance Nevertheless, unconventional hemangiomas of the tip of the nose, lip, and

parotid area are notably slow to involute (18) The appearance of white streaks of fibrosis

on the surface of a lesion is an early sign of regression When the resolution is complete,the affected area may resemble normal skin, but more commonly retains degrees ofatrophy, telangiectases, or redundant anetodermic skin

Occasionally, infantile hemangiomas ulcerate during their proliferative phase withresultant (1) permanent loss of tissue; (2) mutilation of the involved area; (3) recurrent

bleeding; or (4) secondary infection with septicemia (19) The location of the lesion may

introduce singular complications: (1) hemangiomas on the eyelids can impair vision andresult in amblyopia; (2) lesions of the nose, mouth, or upper airway may interfere withfeeding and/or respiration; and (3) hemangiomas on the ear may block the external

auditory canal sufficiently to impair hearing (20,21) In all these cases, directed treatment may be required (22).

A consumptive coagulopathy (Kasabach-Merritt syndrome) is a serious complication

of large hemangiomas This rare syndrome, first described in 1940 (23), consists of

thrombocytopenic purpura and chronic consumptive coagulopathy Hemorrhage is aconsequence of platelet sequestration and the consumption of clotting factors within the

vascular spaces of the hemangioma (24–38) In general, this syndrome is associated with